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Sci Rep 2023 Sep 21;13(1):15760. Epub 2023 Sep 21.

Department of Brain Disease Translational Research, Juntendo University Graduate School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan.

We have previously reported that 12p gain may predict the presence of malignant components and poor prognosis for CNS germ cell tumor (GCT). Recently, 3p25.3 gain was identified as an independent predictor of poor prognosis for testicular GCT. Read More

Folia Neuropathol 2023 ;61(2):121-128

Department of Pathology, The Children's Memorial Health Institute, Warsaw, Poland.

BCOR is expressed in a new brain tumour entity, i.e. 'CNS tumour with BCOR internal tandem duplication' (HGNET BCOR) but not in several other high grade paediatric brain tumours investigated. Read More

Pediatr Blood Cancer 2023 Oct 27;70(10):e30601. Epub 2023 Jul 27.

Division of Hematology and Oncology, St. Louis Children's Hospital, Washington University, School of Medicine in St. Louis, Washington University, St. Louis, Missouri, USA.

Background: The role of neoadjuvant chemotherapy in treating patients with metastatic central nervous system (CNS) germinoma is controversial.

Methods: We compared the relapse-free survival (RFS) of different treatment modalities by performing a meta-analysis using published data. We summarized all data using standard descriptive statistics. Read More

Adv Exp Med Biol 2023 ;1405:421-455

Department of Physical Medicine and Rehabilitation, Taipei Veterans General Hospital, Taipei, Taiwan.

Primary central nervous system germ cell tumors (CNS GCTs) are part of the GCTs in children and adults. This tumor entity presents with geographic variation, age, and sex predilection. There are two age peaks of incidence distribution at the first few months of life and in adolescence. Read More

Adv Exp Med Biol 2023 ;1405:153-173

Department of Neurological Surgery, Columbia University, New York, USA.

Pineal region tumors fall into five broad categories: benign pineal region tumors, glial tumors, papillary tumors, pineal parenchymal tumors, and germ cell tumors. Genetic and transcriptional studies have identified key chromosomal alterations in germinomas (RUNDC3A, ASAH1, LPL) and in pineocytomas/pineoblastomas (DROSHA/DICER1, RB1). Pineal region tumors generally present with symptoms of hydrocephalus including nausea, vomiting, papilledema, and the classical Parinaud's triad of upgaze paralysis, convergence-retraction nystagmus, and light-near pupillary dissociation. Read More

PLoS One 2023 13;18(7):e0288528. Epub 2023 Jul 13.

Department of Nuclear Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.

Purpose: The aim of this study was to investigate the association between methionine (MET) metabolism and endocrine function of the pituitary gland in patients with suprasellar region tumor.

Materials And Methods: Twenty patients with intracranial germinoma were included in this study. Initial staging and all surveillance MET PET/CT scans and comparable serum levels of follicle stimulating hormone (FSH), luteinizing hormone (LH), and thyroid stimulating hormone (TSH) were analyzed. Read More

Cancers (Basel) 2023 May 17;15(10). Epub 2023 May 17.

IIIrd Department of Radiotherapy and Chemotherapy, Maria Sklodowska-Curie National Research Institute of Oncology, 44-102 Gliwice, Poland.

Background: Intracranial germinoma is a rare malignant neoplasm of the central nervous system (CNS) that occurs in children and young adults. The aim of our study was to assess the initial manifestation of the disease, and to find differences in outcomes dependent on time of diagnosis.

Methods: The study group consisted of 35 consecutive patients (adults and children) who were treated for intracranial germinoma with radiotherapy at a tertiary centre, and their data were retrospectively collected. Read More

Clin Oncol (R Coll Radiol) 2023 Sep 19;35(9):571-575. Epub 2023 May 19.

Department of Hematology, Oncology and Radiation Physics, Skåne University Hospital, Lund, Sweden.

There is a lack of clinical protocols for re-irradiation in paediatric central nervous system (CNS) tumours. To fill this void, the Swedish Workgroup of Paediatric Radiotherapy (SBRTG) compiled national guidelines on re-irradiation in paediatric CNS tumours (diffuse intrinsic pontine glioma, ependymoma, germinoma and medulloblastoma). These have been in clinical practice since 2019 in all paediatric radiotherapy centres in Sweden. Read More

Childs Nerv Syst 2023 Sep 18;39(9):2329-2339. Epub 2023 May 18.

Department of Neurology and Neurosurgery, Federal University of Sao Paulo, Rua Napoleão de Barros, 715, 6Th Floor, Zip Code, 04024-002, Sao Paulo, SP, Brazil.

Purpose: This study aimed to identify factors of a worse prognosis among different histological types of pineal region tumors in pediatric patients treat at a single institution in a 30-year period.

Material And Methods: Pediatric patients (151; < 18 years of age) treated between 1991 and 2020 were analyzed. Kaplan-Meyer survival curves were created, and the log-rank test was used to compare the main prognostic factors in the different histological types. Read More

J Neurosurg Pediatr 2023 Aug 5;32(2):184-193. Epub 2023 May 5.

1Department of Neurology and Neurosurgery, Federal University of São Paulo.

Objective: Pineal region tumors account for 2.7%-11% of all CNS tumors in children. In this series, the authors present their surgical results and long-term outcomes from a pediatric pineal region tumor cohort. Read More

J Neurooncol 2023 Apr 11;162(2):443-448. Epub 2023 Apr 11.

Division of Paediatric Haematology-Oncology, Department of Paediatrics, Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada.

Purpose: To determine long-term outcomes of a cohort of children with germinoma treated with chemotherapy and radiation therapy without primary tumor boost even in the absence of complete response to chemotherapy METHODS: This retrospective study analyzed the outcome of patients with germinoma consecutively diagnosed and treated at a tertiary care center from January 2000 to December 2021. MRIs were reviewed by two radiologists, blinded to patient data. Tumor location at diagnosis, tumor response to chemotherapy and at completion of radiation therapy and site of relapse were assessed. Read More

Brain Tumor Pathol 2023 Apr 30;40(2):124-132. Epub 2023 Mar 30.

Department of Brain Disease Translational Research, Juntendo University Graduate School of Medicine, Tokyo, Japan.

Tumor markers in CNS germ cell tumors (GCTs) include human chorionic gonadotropin (HCG) and alpha fetoprotein (AFP), which have significant diagnostic implications, as elevation of either one leads to clinical diagnosis of non-germinomatous GCTs without histopathological confirmation, justifying intensified chemotherapy and irradiation. The current study, based on an international cohort of histopathologically verified GCTs that underwent biopsy (n = 85) or resection (n = 76), sought to better define the clinical role and prognostic significance of tumor markers from serum and CSF in this challenging patient population. We found that HCG was elevated only in cases with a germinoma or choriocarcinoma component, and there existed a clear cut-off HCG value between the two. Read More

J Clin Neurosci 2023 May 22;111:71-77. Epub 2023 Mar 22.

Department of and Pathology, Tianjin Huanhu Hospital, Tianjin 300350, PR China.

Primary suprasellar germinoma (PSG) is a rare malignant tumor of the central nervous system. This study aimed to explore the clinical characteristics, treatment protocol, and prognosis of patients with PSG. This case series retrospectively analyzed the clinical data of patients with PSG in Tianjin Huanhu Hospital diagnosed between January 2016 and December 2021. Read More

NMC Case Rep J 2023 23;10:27-32. Epub 2023 Feb 23.

Department of Neurosurgery, Osaka Medical and Pharmaceutical University, Takatsuki, Osaka, Japan.

Primary germ cell tumors of the central nervous system (CNS) typically occur in the neurohypophysis, hypothalamus, or pineal gland and rarely in the spinal cord. We report a case of a spinal intramedullary tumor, which was first detected on magnetic resonance imaging (MRI) 41 months after the initial symptoms, with a verified pathological diagnosis of germinoma. The initial symptom was an abnormal sensation in the left plantar region that gradually worsened, resulting in severe sensory disturbance, difficulty in standing, and even bladder rectal disturbance. Read More

Jpn J Radiol 2023 Aug 13;41(8):808-829. Epub 2023 Mar 13.

Department of Diagnostic and Interventional Radiology, Graduate School of Medicine, Osaka Metropolitan University, 1-4-3 Asahi-Machi, Abeno-Ku, Osaka, 545-8585, Japan.

Many types of tumors can develop in the pituitary gland. In the recently revised 5th editions of the World Health Organization (WHO) classifications (2021 WHO Classification of Central Nervous System Tumors and the 2022 WHO Classification of Endocrine and Neuroendocrine Tumors), various changes have been made to the tumors other than pituitary neuroendocrine tumor (PitNET)/pituitary adenoma, as well as PitNET. Adamantinomatous craniopharyngioma and papillary craniopharyngioma are now considered separate tumors in the 5th edition of the WHO classification. Read More

Clin Neurol Neurosurg 2023 Apr 24;227:107643. Epub 2023 Feb 24.

Department of Neurological Surgery, School of Medicine and Public Health, University of Wisconsin, Madison, WI, USA. Electronic address:

Intracranial germinomas are most commonly extra-axial germ cell tumors that are predominantly found in the pineal and suprasellar regions. Primary intra-axial midbrain germinomas are extremely rare, with only eight reported cases. Here we present a 30-year-old man who presented with severe neurological deficits, with an MRI that showed a heterogeneously enhancing mass with ill-defined margins in the midbrain, and with surrounding vasogenic edema extending to the thalamus. Read More

World Neurosurg 2023 May 9;173:5-11. Epub 2023 Feb 9.

Department of Neurosurgery, Städtisches Klinikum Karlsruhe, Karlsruhe, Germany; Division of Neurosurgery, University of Foggia, Foggia, Italy.

Obtaining a prompt diagnosis, avoiding indwelling ventriculoperitoneal shunt, and enhancing the predictive value of pathologic examinations are only some of the advantages conferred by a simultaneous third ventriculostomy and tumor biopsy in patients with pineal region tumors. The objective of this study was to retrospectively search the literature on concomitant, single burr hole endoscopic third ventriculostomy (ETV) and tumor biopsy (TB) for pineal region tumors and to analyze the feasibility, surgical safety, and benefits of these 2 combined procedures. Consequently, a comprehensive, systematic literature search was performed in compliance with the updated PRISMA 2020 guidelines within electronic databases MEDLINE/PubMed, EMBASE, PLOS, and Cochrane Library. Read More

Sci Rep 2023 Feb 6;13(1):2073. Epub 2023 Feb 6.

Hunan Key Laboratory of Pediatric Emergency Medicine, Hunan Children's Hospital, The School of Pediatrics, Hengyang Medical School, University of South China, Changsha, Hunan, China.

To strengthen the understanding of the clinical features for CASPR2 neurological autoimmunity in children. A multicenter retrospective and prospective analysis of CASPR2 autoimmunity was conducted. Twenty-six patients were enrolled, including 25 with serum positivity and 3 with cerebrospinal fluid (CSF) positivity; 5 patients were co-positive with anti-NMDAR or anti-GABABR antibodies. Read More

J Radiat Res 2023 Mar;64(2):428-437

Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.

This study investigated the effectiveness and safety of low-dose salvage craniospinal irradiation (CSI) for recurrent germinoma. We retrospectively reviewed long-term tumor control and late adverse effects in 15 recurrent germinoma patients treated at our hospital between 1983 and 2019. Following the first recurrence of germinoma, seven were treated with 24-30 Gy of salvage CSI, three underwent non-CSI, and five were treated with only chemotherapy. Read More

Adv Exp Med Biol 2023 ;1394:85-101

AP-HP, Hôpitaux Universitaires La Pitié Salpêtrière-Charles Foix, Sorbonne Université, Inserm, CNRS, UMR S 1127, Institut du Cerveau-Paris Brain Institute, ICM, Service de Neurologie 2-Mazarin, 75013, Paris, France.

A better understanding of cellular and molecular biology of primary central nervous system (CNS) tumors is a critical step toward the design of innovative treatments. In addition to improving knowledge, identification of the cell of origin in tumors allows for sharp and efficient targeting of specific tumor cells promoting and driving oncogenic processes. The World Health Organization identifies approximately 150 primary brain tumor subtypes with various ontogeny and clinical outcomes. Read More

Eur J Radiol 2023 Feb 20;159:110663. Epub 2022 Dec 20.

University of Illinois, Peoria, IL, USA.

Purpose: To evaluate the effectiveness of diffusion-weighted imaging (DWI) and susceptibility-weighted imaging (SWI) for differentiation between germinoma and other pineal region tumors.

Method: This retrospective study consisted of 72 patients with pathologically proven pineal region tumors between January 2010 and August 2020. Tumors were classified as germinomas (40), non-germinomatous germ cell tumors (11) (NGGCT), pineal parenchymal tumors (10) (PPT), and other types of tumors (11). Read More

Pediatr Blood Cancer 2023 Mar 29;70(3):e30168. Epub 2022 Dec 29.

Department of Paediatric Endocrinology, Royal Marsden NHS Foundation Trust, London, UK.

Aims: This study analysed the clinical features of a cohort of children with intracranial germ cell tumours (IC-GCTs). We retrospectively reviewed timelag between symptoms onset, clinic-radiological findings, diagnosis and outcomes.

Methods: Symptoms at diagnosis were divided into four groups: (1) raised intracranial pressure (RICP); (2) visual impairment; (3) endocrinopathies; (4) other. Read More

Childs Nerv Syst 2023 Feb 20;39(2):505-510. Epub 2022 Dec 20.

Department of Neurosurgery, Busan Paik Hospital, Inje University College of Medicine, Bokji-ro 75, Busanjin-gu, 47392, Busan, Korea.

Background: Intracranial primary germinomas predominantly develop on or near the midline structure in children and young adults and are diagnosed by brain imaging and biopsy. However, if brain imaging and pathology show unusual findings, it becomes difficult to make an accurate diagnosis.

Case Report: Herein, we report the case of a 14-year-old boy who presented with focal dystonia of the fingers as an initial symptom. Read More

Neoplasia 2023 Feb 13;36:100860. Epub 2022 Dec 13.

The Division of Hematology and Oncology, St. Louis Children's Hospital, Washington University, School of Medicine in St. Louis, Washington University, St. Louis, Missouri, USA. Electronic address:

Primary central nervous system (CNS) germ cell tumors (GCT) are a rare heterogenous group of cancers, arising most commonly in the second decade of life. Through several clinical trials conducted around the world by various groups, the treatment approach for CNS GCT has advanced substantially with generally improved overall outcomes. In recent years, the goal of clinical trials has been focused on reduction of the radiotherapy burden and minimization of long-term toxicity. Read More

Neurol Res 2023 Apr 12;45(4):354-362. Epub 2022 Dec 12.

Department of Neurosurgery, Huashan Hospital, Fudan University, Shanghai, China.

Objective: Neuroendoscopic resection supracerebellar infratentorial (SCIT) approach is adequate for some indicated pineal region tumors with the natural infratentorial corridor. We described this full endoscopic approach through a modified 'head-up' park-bench position to facilitate the procedure.

Methods: We reviewed the clinical and radiological data of four patients with pineal region lesions who underwent pure endoscopic tumor resection through the SCIT approach with this modified position. Read More

J Avian Med Surg 2022 Nov;36(3):325-332

Parrish Creek Veterinary Hospital and Diagnostic Center, Centerville, UT 84014, USA.

A 20-year-old male Moluccan eclectus () was presented with a history of falling off the perch, ataxia, unilateral blindness, hyporexia, and progressive lethargy. The eclectus was found to have a leukocytosis (52 × 10 cells/µL), characterized by an absolute and relative heterophilia (42.6 × 10 cells/µL and 82%, respectively), relative lymphopenia (18%), elevated bile acids (88 µmol/L), and elevated triglycerides (236 mg/dL). Read More